Reinterventions and new aortic events after aortic surgery in Marfan syndrome

Great strides have been made in the surgical management of aortic complications of Marfan syndrome (MFS) over the last 5 decades. OBJECTIVES Pre-emptive strategies to manage the aortic complications of Marfan syndrome have resulted in improved life expectancy yet, secondary to the variation of phenotypic expression, anticipating the risk and nature of future aortic events is challenging. We examine rates of new aortic events and reinterventions in a Marfan cohort following initial aortic presentation. METHODS Retrospective cohort study of Marfan patients with aortic pathology presenting to our institution 1998-2018. Patients were grouped according to index event: aortic dissection or root aneurysm. Patients with aortic dissection were classified according to Debakey criteria. Incidence of new aortic events and frequency of reintervention were analysed. RESULTS One hundred and twenty-six aortic procedures were performed in 74 Marfan patients with a median follow-up of 7 years. Forty-seven patients had an index event of root aneurysm and 27 had aortic dissection. Following operative intervention in the aneurysm group, 7 patients developed Debakey III dissections raising the overall number of patients who developed dissection within this cohort to 34. Reinterventions were more frequent in the dissection group with full replacement of the native aorta in 5 patients. CONCLUSIONS After operative intervention on the proximal aorta, a proportion will develop distal pathology. A greater focus on factors contributing to future events, such as mapping genotypes to clinical course, may lead the way for targeted operative techniques and surveillance.

Automated summary

Significant progress has been made in the surgical management of aortic complications of Marfan syndrome (MFS) over the past five decades. However, the varying phenotypic expression complicates the anticipation of future aortic events, highlighting the need for targeted operative techniques and surveillance.